Acute Adrenal Crisis

DRG Category:

644

Mean LOS:

4.9 days

Description:

MEDICAL: Endocrine Disorders with CC

Acute adrenal crisis is a life-threatening endocrine emergency caused by a deficit of glucocorticoids (primarily cortisol) or mineralocorticoids (primarily aldosterone). The anterior pituitary gland produces adrenocorticotropic hormone (ACTH), which causes the adrenal cortex to produce more than 50 steroid hormones. Cortisol and aldosterone are the most physiologically active of these hormones. In primary adrenocortical insufficiency, glucocorticoid and mineralocorticoid function is lost. Acute adrenocortical insufficiency is a difficult diagnosis to make. Acute adrenal crisis rarely occurs without an accompanying serious injury or illness. The diagnosis is difficult because many of the signs and symptoms are nonspecific. However, when left untreated, someone with acute adrenal insufficiency has a very poor prognosis for survival. Any delay in disorder management while waiting for diagnostic confirmation may threaten the person’s life.

Glucocorticoids are cardiac stimulants that activate release of essential vasoactive substances. Without corticosteroids, stress results in hypotension, shock, and death. A deficiency of cortisol produces many metabolic abnormalities, such as decreases in glucose production, protein and fat metabolism, appetite, and digestion. Serious systemic effects include a decrease in vascular tone and a diminished effect of catecholamines, such as epinephrine and norepinephrine. Normally, a body under stress releases corticosteroids. Both the decrease in the vascular tone of the blood vessels and the decreased effect of catecholamines in an individual in an adrenal crisis can cause shock. The deficiency of aldosterone results in profound fluid and electrolyte imbalances: a decrease in sodium and water retention, a decrease in circulating blood volume, and an increase in both potassium and hydrogen ion reabsorption.

Causes

Acute adrenal insufficiency is most commonly caused by acute withdrawal of chronic corticosteroid therapy. It can also occur from immune destruction of the adrenal cortex or adrenal hemorrhage or from infiltration by metastatic carcinoma. Before signs and symptoms appear, at least 90% or more of the adrenal cortex is damaged. The disorders result in deficiencies of both glucocorticoid and mineralocorticoid hormones. Other causes include physiological stress including surgery, infection, burns, sepsis, anesthesia, fluid volume loss, trauma, asthma, hypothermia, alcohol abuse, myocardial infarction, fever, hypoglycemia, pain, and depression.

Genetic considerations

See Adrenal Insufficiency, p. 46.

Gender, ethnic/racial, and life span considerations

Primary adrenocortical insufficiency affects men and women equally, but women are affected two to three times more often by the idiopathic autoimmune form of adrenal insufficiency. In idiopathic autoimmune adrenal insufficiency, the diagnosis is most often discovered in the third to fifth decades of life, but acute adrenal crisis may occur at any age, without regard to gender, and may be associated with developmental or genetic abnormalities. Ethnicity and race have no known effects on the risk for acute adrenal crisis. In children, the most common cause of acute adrenal crisis is an overwhelming infection with Pseudomonas or meningococcal meningitis (also known as Waterhouse-Friderichsen syndrome). In adults, acute adrenal crisis is more commonly associated with hemorrhagic destruction because of anticoagulant therapy or aggravation of adrenal hypofunction during periods of major stress.

Global health considerations

In developed countries, the incidence in Western countries is 50 cases per 1 million persons. No data are available on Eastern countries or many developing nations.

Assessment

History

Elicit a medication history, with particular attention to such medications as corticosteroids, phenytoin, barbiturates, anticoagulants, and rifampin. Note a history of cancer, autoimmune diseases requiring treatment with corticosteroids, or radiation to the head or abdomen. A family history of either Addison’s disease or an autoimmune disease may be important.

Inquire about a recent decrease in appetite, abdominal pain, weight loss, or salt craving. Often in crisis, the patient or family may describe fever, nausea, and vomiting. Determine if the patient has experienced signs and symptoms such as generalized fatigue, apathy, dizziness, weakness, headache, or changes in skin pigmentation. Some patients describe central nervous system effects such as confusion, irritability, psychoses, emotional lability, or even seizures. Women may describe a decreased libido and amenorrhea.

Physical examination

The patient appears to be critically ill with unexplained shock. Because of decreased fluid volume caused by a decrease in water reabsorption, the patient may arrive at the hospital with severe hypotension; tachycardia; decreased cardiac output; weak and rapid pulses; and cool, pale skin. Peripheral pulses may be weak and irregular. Urine output is usually quite low. The blood pressure may be very hard to maintain because a decrease in the catecholamines can result in decreased vascular tone. Other symptoms include nausea, vomiting, flank pain, and hypothermia.

An increase in skin pigmentation (bronze color) is noticeable in European Americans. Areas most often affected include the mucous membranes and areas over joints and scars. A loss of pubic hair may also occur from a decreased level of adrenal androgens.

Psychosocial

Assess the patient’s and significant others’ ability to cope with a critical illness and the presence of a social network to support them.

Diagnostic highlights

Test

Normal Result

Abnormality With Condition

Explanation

Serum cortisol level

6:00 to 8:00 a.m., 5–23 mcg/dL

Decreased

Determines the ability of the adrenal gland to produce glucocorticoids

Interventions

Planning and implementation

Collaborative

Pharmacologic treatment centers on fluid and electrolyte replacement and hormonal supplements (see Pharmacologic Highlights box). Fluid replacement with dextrose- and sodium-containing solutions allows for correction of hypovolemia and hypoglycemia. As much as 5 L or more of fluid may be needed to maintain an adequate blood pressure, circulation, and urine output and to replace the fluid deficit. Correct sodium, potassium, calcium, and glucose abnormalities. Always treat the underlying problem that precipitated the crisis.

Pharmacologic highlights

Medication or Drug Class

Dosage

Description

Rationale

Dexamethasone

4 mg IV

Corticosteroid

Substitution therapy in deficiency state; drug does not interfere with serum cortisol assay; dangerous to delay glucocorticoid replacement while awaiting ACTH stimulation test results; drug does not have mineralocorticoid activity and therefore needs to be accompanied by fluid and electrolyte replacement

Independent

During the initial hours of managing a patient with acute adrenal crisis, the first priority is to maintain airway, breathing, and circulation. Patients who receive large amounts of room-temperature fluids are at risk for hypothermia. Keep the temperature of the room warm and the bed linens dry. If possible, keep the patient fully covered. During massive fluid replacement, administer warmed (body-temperature) fluids if possible.

Teach the patient on corticosteroids about the medication and the need to continue to take it until the physician tapers the dose and then finally discontinues it. Explain the symptoms of adrenal crisis to any patient who is undergoing anticoagulant therapy. Explain the effects of stress on the disease and the need for adjustment of medications during times of stress.

Patients with altered tissue perfusion require frequent skin care. If the patient is immobile, perform active and passive range-of-motion exercises at least every 8 hours. Encourage coughing and deep breathing to limit the complications from immobility. Provide small, frequent meals and make referrals to the dietitian early in the hospitalization.

Evidence-Based Practice and Health Policy

Prevention of acute adrenal crisis is a priority to avoid development of life-threatening complications.

Primary prevention recommendations include advising patients to double their regular hydrocortisone replacement doses during periods of illness and increased stress.

Secondary prevention recommendations include supplying patients with a medical alert bracelet and facilitating early admission and emergency treatment with parenteral administration of hydrocortisone as soon as patients recognize an impending crisis.

Discharge and home healthcare guidelines

patient teaching.

Teach the patient and significant others about the disease and the factors that aggravate it. Provide suggestions about rest and activity and stress reduction. Explain the signs and symptoms that may lead to crisis.

prevention.

Identify the stressors and the need to increase medication during times of stress. Teach the patient and family when the physician needs to be notified.

medications.

Teach the patient the name, dosage, action, and side effects of drugs and the need to continue using them for life. Provide written instruction about medications and follow-up physician’s appointments.

Levothyroxine is contraindicated in patients with uncorrected adrenal insufficiency since thyroid hormones may precipitate an acute adrenal crisis by increasing the metabolic clearance of glucocorticoids.

In the most severe form of the disorder, called salt-wasting CAH, or SWCAH, the body can't maintain a proper fluid balance, setting up a lifelong possibility of acute adrenal crisis characterized by dehydration, vomiting, diarrhea, low blood sugar, shock and even death.

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